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進行性 筋性 筋萎縮症

Progressive Muscular dystrophy with Involvement of the Heart

대한의학협회지 1962년 5권 3호 p.58 ~ 65
오신중 (  ) - 국립의료원 신경과

Abstract


Since the description of progressive muscular dystrophy (P.M.D.) in 1852 by Meryon (1), there .have been many reports of the various clinical
features of this disease. Only few observers have, However, reported the occurrence of heart disease in patients with, P.M.D. Judging from these reports it would seem that the finding of a specific muscular lesion in the heart of patients with P. M. D; is much more common than has hitherto been believed.
Boas and Lowenberg (2), summarized the work of. Meerwein (35, who studied all the 480 cases of progressive muscular dystrophy reported up to 1961. Of these cases, 89 presented some abnormality of the heart or pulse. He divided these latter patients into 4 groups; Group I consisted. of 13 patients with abnormal pulse rates but no cardiac murmurs or evidence of cardiac enlargement, Group 11, 12 patients with definite murmurs but no enlargement ´of the heart; Group III, 49 patients who showed cardiac enlargement; Group IV, 15 autopsied patie nt s who showed definite lesions of the myocardium. The lesions here have a striking similarity to those found in the skeletal muscles but are usually of a milder degree.
Rubin and Buchberg (4), in a study of 33 cases of progressive muscular dystrophy reported: specific cardiac musular changes in 50% of their patients. Clinically their cardiac symptoms fall into 3 categories: QQ patients who develop congestive heart failure; 2 patients with arrhythmia or tachycardia; and G patients with ECG manifestations only. Weisenfeld and Messinger´s analysis of 44 cases (5) showed that 85% had clinical manifestations of dysfunction of the cardiovascular system. Clinical features: The most common single abnormality was the occurrence of tachycardia, occasionally of the paroxysmal type. This was observed in 50% of Weisenfeld and Messinger´s series (5). Other physical manifestations were found in 35% of the patients although none of the individual findings occurred with significant frequency. These included congestive heart failure, cardiac murmur, disturbance of rhythm, and cardiac enlargement. Roentgenologically, the latter is characterized by a generalized -hypertrophy (ordilataion) of the chambers. Confusing signs and symptoms may be seen in other organs, due to emboli arising from either side of the heart(6).Since cardiac involvement is stated to be so common in this condition, it is interesting to consider why it is not more often recognized clinically. One reason may be that in the late stage of P.M.D. many patients are bedridden and do a minimum of exercise so that indications of cardiac decompensation such as exertional dyspnea and easy fatigability are not as evident. Also, some of the signs and symptoms in this disease, such as dyspnea, dependent edema, and cyanosis of the extremities, which may be cardiac in origin, may be (undefined)

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